Carlos Wolff

Plasma and urinary levels of thiobarbituric acid reactive substances (TBAR) were determined in 24 hyperthyroid patients, 19 hypothyroid subjects, 35 controls, and 17 hyperthyroid patients before and after propylthiouracil (PTU) treatment (400 mg/day for 2-3 months), as indexes of lipid peroxidation. These measurements were carried out together with t-butyl hydroperoxide (t-BHP)-induced oxygen uptake and visible chemiluminescence in erythrocytes as functional tests related to the antioxigenic capacity of cells. Hyperthyroid patients exhibited increased levels of plasma and urinary TBAR compared to controls. Erythrocyte suspensions from hyperthyroid patients showed, compared to controls, higher rates of oxygen consumption with shorter induction periods upon addition of t-BHP, together with 142% and 75% increases in basal and t-BHP-induced chemiluminescence, respectively. Levels of TBAR in untreated hyperthyroid patients in plasma (16.2 +/- 1.3 pmol/mg of protein) and urine (15.9 +/- 1.5 nmol/mg of creatinine) were decreased after PTU treatment (Plasma, 9.5 +/- 0.7, p less than 10(-4); urine, 7.8 +/- 0.9, P less than 10(-5) to values not significantly different from those of the control group (plasma, 10.3 +/- 0.6; urine, 7.9 +/- 0.7). Compared to control, elevated rates of oxygen uptake induced by t-BHP, basal and t-BHP-induced chemiluminescence in erythrocyte suspensions from untreated hyperthyroid patients were reverted to normal by PTU, while decreased induction period (T0) values were enhanced. Determination of these lipid peroxidative parameters in hypothyroid patients revealed no significant changes over control values, excepted t-BHP-induced chemiluminescence in erythrocytes that was diminished. These data indicate that hyperthyroidism is associated with a pro-oxidant condition characterized by an enhancement in circulating and urinary lipid peroxidative indexes, which is suppressed by PTU treatment. It is suggested that this condition might reflect an oxidative stress at cellular level in tissues which are target for thyroid hormone action with a calorigenic response.

We report a 67 years old woman admitted to the hospital for the study of a cholestatic jaundice and massive hepatomegaly. On admission, the patient did not have liver failure. During hospital stay, the patient experienced a progressive deterioration of liver function and a monoclonal gammopathy was detected. An IgG Kappa myeloma-was diagnosed. A fine needle liver biopsy disclosed the presence of amyloid. The patient developed acute liver failure and died three weeks after admission.

We report a 67 years old woman admitted to the hospital for the study of a cholestatic jaundice and massive hepatomegaly. On admission, the patient did not have liver failure. During hospital stay, the patient experienced a progressive deterioration of liver function and a monoclonal gammopathy was detected. An IgG Kappa myeloma-was diagnosed. A fine needle liver biopsy disclosed the presence of amyloid. The patient developed acute liver failure and died three weeks after admission.

1. Sulphobromophthalein (BSP) retention and liver glutathione levels were studied in uncomplicated hyperthyroid patients. 2. BSP retention was increased in 52.5% of the subjects at admission (n = 40) and in 28% of the cases after 3 months of propylthiouracil treatment (300-400 mg/day) (n = 25). 3. Hepatic levels of glutathione were measured in six patients and significant inversed power correlation with BSP retention was observed (r = 0.968, P less than 0.001). 4. These data support the contention that the alteration of the BSP retention observed in hyperthyroidism could be due to a decreased availability of hepatic glutathione for conjugation.

Plasma and urinary levels of thiobarbituric acid reactive substances (TBAR) were determined in 24 hyperthyroid patients, 19 hypothyroid subjects, 35 controls, and 17 hyperthyroid patients before and after propylthiouracil (PTU) treatment (400 mg/day for 2-3 months), as indexes of lipid peroxidation. These measurements were carried out together with t-butyl hydroperoxide (t-BHP)-induced oxygen uptake and visible chemiluminescence in erythrocytes as functional tests related to the antioxigenic capacity of cells. Hyperthyroid patients exhibited increased levels of plasma and urinary TBAR compared to controls. Erythrocyte suspensions from hyperthyroid patients showed, compared to controls, higher rates of oxygen consumption with shorter induction periods upon addition of t-BHP, together with 142% and 75% increases in basal and t-BHP-induced chemiluminescence, respectively. Levels of TBAR in untreated hyperthyroid patients in plasma (16.2 +/- 1.3 pmol/mg of protein) and urine (15.9 +/- 1.5 nmol/mg of creatinine) were decreased after PTU treatment (Plasma, 9.5 +/- 0.7, p less than 10(-4); urine, 7.8 +/- 0.9, P less than 10(-5) to values not significantly different from those of the control group (plasma, 10.3 +/- 0.6; urine, 7.9 +/- 0.7). Compared to control, elevated rates of oxygen uptake induced by t-BHP, basal and t-BHP-induced chemiluminescence in erythrocyte suspensions from untreated hyperthyroid patients were reverted to normal by PTU, while decreased induction period (T0) values were enhanced. Determination of these lipid peroxidative parameters in hypothyroid patients revealed no significant changes over control values, excepted t-BHP-induced chemiluminescence in erythrocytes that was diminished. These data indicate that hyperthyroidism is associated with a pro-oxidant condition characterized by an enhancement in circulating and urinary lipid peroxidative indexes, which is suppressed by PTU treatment. It is suggested that this condition might reflect an oxidative stress at cellular level in tissues which are target for thyroid hormone action with a calorigenic response.

The purpose of this study is to determine whether the use of the Goretex Pantaloon cast liner for children's spica casts reduces urine excoriation of the skin under the cast and reduces the incidence of unplanned cast changes necessitated by cast soilage. Between 1988 and 1993, 72 consecutive children who had 147 hip spica casts applied were followed. The first 36 patients had 77 spica casts applied without the Goretex Pantaloon, and the last 36 patients had 70 spica casts with a Goretex liner. In the group of patients without Goretex, 22% of casts were associated with urine excoriation of the skin compared to 1.4% in the group of patients who had a Goretex liner applied. This was statistically significant (p = 0.0005). The incidence of unnecessary cast changes due to soiling of the cast was 14% in the non-Goretex group compared to 2.9% in the Goretex group (p = 0.05). The use of the Goretex liner in our series resulted in a savings of $38 per cast including the cost of the liners ($75). The Goretex Pantaloon cast liner used for children's spica casts prevents urine excoriation of the skin and is cost effective.

BACKGROUND: Tc99m DMSA (dimercaptosuccinic acid) scintigraphy has a high sensitivity for the detection of cortical kidney damage.AIM: To evaluate the Tc99m DMSA renal scintigraphy in children with a first episode of acute pyelonephritis and its association with laboratory parameters, kidney ultrasound and vesicoureteral reflux.PATIENTS AND METHODS: We studied 143 children (age range 8 days, 12 years, 66% female) hospitalized with the clinical diagnosis of acute pyelonephritis (first episode) with a positive urine culture and a renal scintigraphy performed within seven days of diagnosis. DMSA was considered the gold standard for the detection of cortical lesions. Its results were related to the presence of fever, C-reactive protein (CRP), erythrocyte sedimentation rate (VHS), white blood count (WBC), ultrasound examination and vesicoureteral reflux.RESULTS: Seventy nine percent of the population had an abnormal DMSA scan. There were no differences between sex, age and laboratory parameters in children with normal or abnormal DMSA scans, except for CRP (p <0.005). Ultrasound was coincident with the scan in 32% of patients. Eighteen percent had vesicoureteral reflux.CONCLUSIONS: There is a high proportion of abnormal DMSA scans in children with a first episode of acute pyelonephritis.

In patients with porphyria cutanea tarda (PCT), hepatic iron accumulation associated to hereditary hemochromatosis (HH) could play a role in the etiology and in the clinical expression of the disease. The H63D and C282Y mutations of the HFE gene frequency were studied in a PCT group of patients and compared with the frequency observed in a group of volunteer blood donors. PCT patients were cataloged as hereditary or acquired PCT carriers, whether or not they presented uroporphyrinogen decarboxilase gene mutations. Fifty percent of PCT patients were carriers of the disease&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s genetic type. Such percentage is significantly higher than what other authors have previously informed. H63D and C282Y mutations were present in 23% and 2.4% of the volunteer blood donors, respectively. Similar frequencies were informed by others authors in Chilean white ethnic populations, and also in Spaniard and Argentinean populations, but significantly higher than that observed in Chile&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s Araucanean aboriginal population. Probably the frequency of H63D and C283Y mutations are related to the Spaniard ascendancy dominance of Chile&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s white ethnic population. The frequency of HFE gene mutations in PCT patients was not different than what was observed in volunteer blood donors. Similarly, there was no statistical difference in the frequency of these mutations among patients with acquired or genetic PCT disease. With the obtained results, it is not possible postulate an association between PCT and the hereditary hemochromatosis of HFE gene mutations carrier conditions.

Resumen:Debido a la importancia que posee la cuantificación precisa del tamaño del infarto miocárdico (IM), se estudió la concordancia entre su tamaño determinado mediante cintigrafía miocárdica SPECT utilizando un programa de cuantificación automático independiente del operador y Ecocardiografía bidimensional, en 24 pacientes con antecedentes de IM antiguo (más de 1 mes). Los IM se dividieron en 3 categorías: grandes, medianos y pequeños. Se obtuvo concordancia estadísticamente significativa, aunque de grado moderado, entre ambas técnicas. Concluimos que el método cintigráfico asociado a este programa de cuantificación automático se compara bien a la Ecocardiografía, con la ventaja para el primero de ser operador independiente. Es posible que el grado de concordancia hubiese podido ser incluso mayor, si se hubiese usado en Ecocardiografía análisis con doppler tisular en forma sistemática.AbstractDue to the importance of an accurate quantification of the myocardial infarction size, ...

Variegate porphyria (VP) results from a hereditary deficiency of protoporphyrinogen oxidase (PPOX) that is transmitted in an autosomal dominan fashion. The diagnosis is based on the clinical symptoms and is confirmed biochemically. Sometimes, however, these diagnostic tools reveal limitations in establishing the definitive diagnosis of the prevailing type of acute porphyria. In these patients, molecular genetic analyses can be useful. We performed molecular genetic studies in 13 Chilean families by PCR amplification of the PPOX gene, conformation sensitive gel electrophoresis, and automated DNA sequencing. In five symptomatic patients from different families, respectively, the biochemical data confirmed the diagnosis of VP. In seven other families, however, the biochemical studies were not conclusive. Furthermore, the original biochemical analysis in one clinically severely affected patient from a further family even suggested the diagnosis of erythropoietic protoporphyria (EPP). Be...

Acute pyelonephritis in children can leave a kidney scar that eventually can lead to hypertension or renal failure. 99mTc-dimercaptosuccinic acid renal scintigraphy (RC DMSA) is a widely accepted technique to assess children with acute pyelonephritis. To evaluate the presence of residual kidney scars detected through RC DMSA, in children with a first episode of acute pyelonephritis. Clinical records of children with a first episode of acute pyelonephritis that were assessed within seven days of the episode with RC DMSA were reviewed. Children were considered eligible if they did not have a new episode of acute pyelonephritis and a second RC DMSA, one year after the first episode, was performed. The presence or absence of a renal scar after one year was associated to demographic, scintigraphy and laboratory variables. Fifty nine children, aged 1 month to 10 years, 35 females, were studied. Thirty nine percent had a renal scar in the scintigraphy perfomed after one year of follow up. ...

Tc99m DMSA (dimercaptosuccinic acid) scintigraphy has a high sensitivity for the detection of cortical kidney damage. To evaluate the Tc99m DMSA renal scintigraphy in children with a first episode of acute pyelonephritis and its association with laboratory parameters, kidney ultrasound and vesicoureteral reflux. We studied 143 children (age range 8 days, 12 years, 66% female) hospitalized with the clinical diagnosis of acute pyelonephritis (first episode) with a positive urine culture and a renal scintigraphy performed within seven days of diagnosis. DMSA was considered the gold standard for the detection of cortical lesions. Its results were related to the presence of fever, C-reactive protein (CRP), erythrocyte sedimentation rate (VHS), white blood count (WBC), ultrasound examination and vesicoureteral reflux. Seventy nine percent of the population had an abnormal DMSA scan. There were no differences between sex, age and laboratory parameters in children with normal or abnormal DM...

... Carlos Wolff F 1a , Pilar Durruty A 1,4a , Jaime Espinoza R 2a , Soledad Ripamonti Z 3b , Jaime Díaz C 4 . ... 19. Liew M, Hoffmann M, Koening W, Brenner H, Rothebacher D. Genotype and plasma concentration of cystatin C in patient with coronary heart disease and risk for ...

... 4. Stratton IM, Adler AI, Neil HA, Matthews DR, Manley SE, Cull CA et al. ... Hermansen K, Colombo M, Storgaard H, Oster-gaard A, Kolendorf K, Madsbad S. Improved postprandial glycemic control with biphasic insulin aspart relative to biphasic insulin lispro and biphasic human ...

... Se estima que haber tenido una depresión mayor es contraindicación del tratamiento. ... [ Links ]. 3. HERRERO C, VICENTE A, BRUGUERA M, ERCILLA MG, BARRERA JM, VIDAL J ET AL. Is hepatitis C virus infection a trigger of porphyria cutanea tarda? ...

A 6-mo-old, male African hedgehog (Atelerix albiventris) presented with a history of pink urine and demonstrating pink-colored teeth and mild hepatomegaly on examination. Urinalysis revealed no physical, chemical, or cellular abnormalities other than a pink color and fluorescence under ultraviolet light (UV). Also under UV, intense fluorescence of teeth, feet, and spines was noted. Porphyria was suspected. Spectrophotometric evaluation of urine showed extremely elevated levels of copro- and uroporphyrins. Analysis of the urine by thin-layer chromatography showed an abnormal pattern of excreted porphyrin intermediates. Urine high-performance thin-layer chromatography showed that excreted porphyrins were 90-95% of the type-I isomeric form, suggestive of congenital erythropoietic porphyria.

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Mutations of hemochromatosis gene in volunteer blood donors and Chilean porphyria cutanea tarda patients. In patients with porphyria cutanea tarda (PCT), hepatic iron accumulation associ- ated to hereditary hemochromatosis (HH) could play a role in the etiology and in the clinical expression of the disease. The H63D and C282Y mutations of the HFE gene frequency were studied in a PCT

The porphyrias comprise a group of fascinating disorders resulting from predominantly inherited as well as acquired deficiencies of one of the eight enzymes along the pathway of heme biosynthesis. On the basis of clinical aspects, the different types of porphyrias can be classified in acute and non-acute forms. However, an exact classification is often difficult since the porphyrias might reveal unspecific clinical symptoms and/or overlapping biochemical features. In particular, this is true for the acute porphyrias which can present with life-threatening acute neurovisceral attacks that require immediate medical intervention. Due to the multiple facets of these disorders, the diagnosis and treatment of the acute porphyrias should always imply a close interdisciplinary collaboration to serve patients and their families most effectively.

Variegate porphyria (VP; OMIM 176200) is characterized by a partial defect in the activity of protoporphyrinogen oxidase (PPO), the seventh enzyme of the porphyrin-heme biosynthetic pathway. The disease is usually inherited as an autosomal dominant trait displaying incomplete penetrance. In an effort to characterize the spectrum of molecular defects in VP, we identified 3 distinct mutations in 6 VP families from Chile by PCR, heteroduplex analysis, automated sequencing, restriction enzyme digestion and haplotyping analysis. The mutations consisted of 2 deletions and 1 missense mutation, designated 1239delTACAC, 1330delT and R168H. The occurrence of the missense mutation R168H had been reported previously in American, German and Dutch VP families, suggesting that this may represent a frequent recurrent mutation. Interestingly, the mutation 1239delTACAC was found in patients from 4 unrelated families living in different parts of Chile, suggesting that it might represent a common mutation in Chile. Haplotype analysis using 15 microsatellite markers which closely flank the PPO gene on chromosome 1q22, spanning approximately 21 cM, revealed the presence of R168H on different haplotypes in 6 VP patients from 3 unrelated families. In contrast, we found the occurrence of 1239delTACAC on the same chromosome 1 haplotype in 11 mutation carriers from 4 unrelated families with VP. These findings are consistent with R168H representing a hotspot mutation and 1239delTACAC existing as a founder mutation in the PPO gene. Our data comprise the first genetic studies of the porphyrias in South America and will streamline the elucidation of the genetic defects in VP patients from Chile by allowing an initial screening for the founder mutation 1239delTACAC.

O objetivo desse livro é avaliar o impacto da liberalização comercial e das instituições sobre o crescimento econômico nos países do MERCOSUL no período 1990-2005. O estudo está dividido em duas partes. A Parte A é dedicada à uma avaliação dos efeitos da abertura comercial e dos indicadores de governança no desempenho do comércio e da produtividade do MERCOSUL e está composta por uma análise do bloco e de estudos de caso de cada um dos países membros. A Parte B analisa as instituições de defesa comercial e de política de concorrência dos países sócios do MERCOSUL, tendo como referência a experiência internacional nessas áreas. RED MERCOSUR